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Case | Age at onset (months) | Age at death (months) | Age alive (years) | Sex | Primary clinical features and followup (f/u) | Echo data | Noncompaction LV (LVNC) | LV function FS % |
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A6 | 2 | 2 | | f | Respiratory distress, CHF, cardiogenic shock | LV dilatation LVEDD-39 mm Noncontracting LV | Noncompaction LV | FS < 10% |
A7 | 8 | | 14 months psychomotor development adequate to age | m | Cardiomegaly in X-ray asymptomatic f/u-asymptomatic | LV dilatation LVEDD-36 mm Mild LVH, mild MVI Mild LV dysfunction | | 25% |
A8 | 4 | | 2 y, adequate psychomotor development to age | f | Respiratory distress f/u-frequently hospitalized due to CHF | LV dilatation LVEDD-44 mm LV dysfunction Moderate LVH | | 15–17% |
A9 | 2 | 11 | | m | Respiratory distress f/u-frequently hospitalized due to CHF | LV dilation LVEDD-44 LV dysfunction Moderate MVI Moderate LVH | | 12–15% |
A10 | 3 | 5 | | f | Respiratory distress, CHF | LV dilation LVEDD-42 mm Noncontracting LV | | 11–13% |
A11 | 2 | 2 | | f | Respiratory distress, CHF | LV dilation LVEDD-37 mm Noncontracting LV | Noncompaction LV | FS < 10% |
A12 | 5 | | 7 y, normal school performance | m | Mild respiratory distress f/u-exercise intolerance | LV dilation LVEDD-46 LV dysfunction Mild MVI Mild LV hypertrophy | | 23–25% |
A13 | 6 | 6 | | m | Respiratory distress, CHF cardiogenic shock | LV dilatation LVEDD-42 mm Noncontracting LV | Noncompaction LV | FS < 10% |
B1 | 33 W of gestation | 2 | | m | Respiratory distress, CHF cardiogenic shock | LV dilatation LVEDD-36 mm Non contraction LV | Noncompaction LV | FS < 10% |
B2 | 1 | | 8 y, normal school performance | f | Respiratory distress frequently hospitalized at one year-old, f/u-exercise intolerance | LV dilatation LVEDD-50 mm LV dysfunction Mild MVI, mild LVH | | 22-23% |
B3 | 3 | 8 | | f | Respiratory distress CHF | LV dilatation LVEDD-44 mm Severe LV dysfunction | Noncompaction LV | 13% |
B4 | 32 W of gestation | 1 | | f | Respiratory distress, CHF, sudden death at home | LV dilation LVEDD-33 mm Noncontracting LV | Noncompaction LV | FS < 10% |
B5 | 32 W of gestation | | 14 m, walked at age12 m | m | Respiratory distress f/u-frequently hospitalized due to CHF | LV dilatation LVEDD-43 mm Mild MVI Moderate to severe LV dysfunction | Noncompaction LV | 12–18% |
C1 | 4 | 4 | | m | Respiratory distress CHF, cardiogenic shock | LV dilatation LVEDD-43 mm Non contraction LV | | FS < 10% |
D1 | 8 months | | 11 y, normal school performance | m | At presentation-respiratory distress f/u-exercise intolerance | LV dilatation LVEDD-48 mm Mild LVH, mild MVI Mild LV dysfunction | | 24–26% |
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